title: Severe intrahepatic cholestasis, erythrocytosis and hypoglycemia: unusual presenting features of systemic AL amyloidosis.
abstract: We report the case of a 68-year-old African woman who presented with jaundice, hepatomegaly and anasarca. Clinical investigation disclosed severe intrahepatic cholestasis, nephrotic syndrome, erythrocytosis and hypoglycemia. Diagnosis of systemic AL amyloidosis was established by percutaneous liver biopsy. Bone marrow biopsy showed 32% of myeloma cells. The patient started treatment with melphalan and prednisolone, but liver function deteriorated and she died in hepatic failure complicated by septic shock three weeks after the diagnosis. We present possible explanations for the unusual clinical and laboratory findings, which required a multidisciplinary approach and posed challenging problems in differential diagnosis and management.
authors.affiliation: Department of Gastroenterology, Liver Unit, Hospital de Santa Maria, E.P.E. Avenida Professor Egas Moniz, Lisbon, Portugal. saragferreira@netcabo.pt
authors.forename: Sara
authors.initials: S
authors.lastname: Ferreira
authors.forename: Cilénia
authors.initials: C
authors.lastname: Baldaia
authors.forename: Narcisa
authors.initials: N
authors.lastname: Fatela
authors.forename: Fernando
authors.initials: F
authors.lastname: Ramalho
authors.forename: Maria Adília
authors.initials: MA
authors.lastname: Costa
authors.forename: Delfina
authors.initials: D
authors.lastname: Brito
authors.forename: Graça
authors.initials: G
authors.lastname: Esteves
authors.forename: Estela
authors.initials: E
authors.lastname: Monteiro
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis / Q000209:etiology*; D003937:Diagnosis, Differential; D004906:Erythrocyte Count; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007003:Hypoglycemia / Q000175:diagnosis / Q000209:etiology*; D019152:Paracentesis; D011086:Polycythemia / Q000175:diagnosis / Q000209:etiology*; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed